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Acute forearm compartment syndrome associated with HIV-induced thrombocytopenia
Acute Forearm Compartment Syndrome Associated With HIV-induced Thrombocytopenia Sanjay S. Desai, MD, Clare Kearns McCarthy, MD, Anita Kestin, MD, Jeffrey N. Metzmaker, MD, Worcester, MA
Acute compartment syndrome is most commonly associated with an upper extremity fracture; however, nontraumatic cases of acute forearm compartment syndrome associated with hemophilia’-’ and leukemic infiltrate@ have been documented. We describe a case of acute compartment syndrome following relatively minor trauma in a patient with human immunodeficiency virus (HIV)-associated thrombocytopenia.
Case Report A 25year-old Hispanic man, inmate at the county house of correction, bumped his left elbow and forearm lightly against a door 3 hours prior to presenting to the emergency department. He was complaining of moderate pain and swelling in the left forearm and elbow. He had no paresthesias at this time. He initially denied the use of any prescription or overthe-counter medications. Upon further questioning he admitted to prior intravenous drug use; an assay for antibody to the HIV had apparently been found to be positive 6 months prior to admission. He denied a history of joint pain, joint swelling, fever, cough, dysuria, rash, or unusual bleeding. On physical examination, he had significant swelling of the From the Department of Orthopaedics and Physical Rehabilitation, University of Massachusetts Medical Center and Department of Hematology, Medical Center of Central Massachusetts/ Memorial Hospital, Worcester, MA. Received for publication July 2, 1992; accepted in revised form March 15, 1993. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Sanjay S. Desai, MD, Department of Orthopaedics and Physical Rehabilitation, University of Massachusetts Medical Center, 55 Lake Avenue North, Worcester, MA 01655.
elbow and proximal forearm, but these were not tense. He had no pain on passive stretch of the fingers and had normal sensation to light touch in the median, ulnar, and radial nerve distribution in the left hand. There were no motor deficits in the left hand or forearm. He was afebrile. X-ray films revealed no evidence of fracture or dislocation in the elbow or forearm. Initial laboratory data were hematocrit, 38%; white blood cell count, 4900; and platelet count, 18,000. The prothrombin time was 12.1 seconds and the activated partial thromboplastin time was 41.6. The patient was admitted to the orthopaedic surgery service for splinting, frequent neurovascular checks, moderate elevation of the limb, and a hematology consultation. Approximately 18 hours after admission the patient began complaining of increased pain in the forearm and decreased sensation in the fingers of the left hand. On physical examination the forearm was noted to be more tense and the patient had increased pain on passive extension of the fingers. The anterior forearm compartment pressure, measured with a slit catheter, was in the 32-34 mmHg range. The patient was admitted to the surgical intensive care unit for closer monitoring and the slit catheter was left in place. Serial measurements over the next few hours showed anterior compartment pressures in the 28-30 mmHg range. The patient experienced some decrease in pain. Antinuclear antibody was found to be negative. A screen for disseminated intravascular coagulation was also negative, with a normal fibrinogen level and no elevation of fibrin split products. A screen for factor XIII level (clot solubility in 5 M urea) was also found to be normal. Because the patient was found to have a minimal prolongation of the partial thromboplastin time, factors VIII, IX, and XII were The Journal of Hand
Surgery
865
866
Desai et al.
i Acute Compartment
Syndrome
and HIV
measured and found to be normal. The factor XI level was found to be minimally decreased (54%). An equal mixture of patient and normal plasma demonstrated full correction of the activated partial thromboplastin time, arguing against the presence of a circulating inhibitor. The patient’s activated partial thromboplastin time returned to normal over the course of his stay in the hospital and remained normal thereafter. A presumptive diagnosis of HIV-related immune thrombocytopenia was made, and the patient was started on 60 mg of prednisone per day. Over the next 24 hours pain and paresthesias were markedly improved, and the last slit catheter pressure measured prior to discontinuing the catheter was 21 mmHg. The patient continued to improve over the next 3 days. At the time of discharge the patient’s platelet count was up to 142,000. He was discharged on 60 mg of prednisone per day. The patient had intermittent bouts of forearm swelling over the next several weeks, which correlated with waxing and waning of the platelet count. He required readmission for limb swelling but had no paresthesias or significant pain. In spite of continued steroid and Danazol therapy, the patient’s platelet count continued to be low. At short-term follow-up 6 weeks after his admission, he had some elbow stiffness but no evidence of ischemic contracture. Three months after his initial presentation he underwent splenectomy for refractory HIV-associated thrombocytopenia. He was lost to follow-up after this procedure.
Discussion Immune thrombocytopenia is a relatively common complication of intravenous drug use and can occur both in HIV-positive and HIV-negative subpopulations.’ The mechanism responsible for thrombocytopenia in this setting is unclear, although evidence has been found to implicate immune complexes’ and 7s IgG in the pathogenesis of narcoticassociated immune thrombocytopenic purpura.‘-” Compartment syndrome is a well-documented complication of hemophilia.‘-5 It is, however, an uncommon complication of qualitative or quantitative platelet disorders. For this reason, an extensive search for a second hemostatic defect was undertaken. The laboratory studies showed no evidence to support the presence of disseminated intravascular coagulation, coagulation factor deficiency, or a circulating anticoagulant. Definitive diagnosis in this case was hampered because the patient denied a history of intravenous drug abuse. However, consultation with a hematolo-
gist and further questioning of the patient yielded the correct diagnosis. The diagnosis of HIV-induced thrombocytopenia should be considered in young men with a low platelet count, whether or not the patient admits to any known risk factors. Platelet transfusion is generally not effective in raising the platelet count in immune-mediated thrombocytopenia. ‘* The main treatment modalities available include corticosteroids, intravenous immunoglobulin, Danazol, and splenectomy. The mechanism of action of these treatments is poorly understood. Currently accepted theories suggest that corticosteroids exert their effect in thrombocytopenia by reducing macrophage uptake of antibody-coated platelets by inhibiting F, receptor-mediated reticuloendothelial system function. Danazol is an androgenic steroid that is thought to decrease the number of F, receptors on monocytes and macrophages, thereby decreasing their ability to phagocytose altered platelets. Splenectomy eliminates a common site of platelet destruction.13 Most of these mechanisms are hypothetical. In this patient corticosteroids were useful as an initial measure to increase platelet count. It is possible that the patient’s symptoms improved spontaneously rather than due to the prednisone. However, throughout his subsequent clinical course there was a close correlation between his platelet count, forearm symptoms, and prednisone dose. That is, whenever his prednisone dose was decreased, his symptoms became worse and his platelet count decreased. In the long term, however, corticosteroids and Danazol were not effective in maintaining an adequate platelet count and the patient underwent splenectomy . We do not have unequivocal evidence that bleeding in the forearm was the cause of the patient’s symptoms. Short of surgery, magnetic resonance imaging may have been helpful in defining the pathology of increased compartment pressure in this situation. While there was minor trauma reported by the patient, his platelet count was below the accepted threshold (20,000) for spontaneous bleeding to occur. i4 While 30 mmHg is generally considered the threshold for fasciotomy in an acute compartment syndrome, it is a relative indication that must be tempered with the trend of symptoms and signs, as well as the trends of compartment pressures.i5 With steroids, rest, splinting, and mild elevation, the patient’s symptoms improved and compartment pressures showed a downward trend. This was fortunate as surgery on a thrombocytopenic patient, who may be actively bleeding, is a high-risk procedure. To our knowledge, this is the first reported case of
The Journal of Hand Surgery I Vol. 18A No. 5 September 1993
compartment syndrome related to HIV-associated thrombocytopenia. We recommend a high index of suspicion for HIV-induced thrombocytopenia in the patient with a history of minor trauma, compartment syndrome, and a low platelet count. We suggest prompt hematologic consultation and institution of medical therapy, along with rest, splinting, and monitoring of compartment pressures. We do not have experience in the surgical management of this entity, however, the compartment pressures and patient’s symptoms and signs could warrant surgical intervention. We recommend performing the fasciotomy in a standard fashion with a pneumatic tourniquet, meticulous hemostasis, and a bulky postoperative dressing with a splint. We also recommend close communication between the hand surgeon, hematologist, and general surgeon. When feasible, maximal pharmacologic therapy as outlined should be undertaken to increase the platelet count before fasciotomy. If necessary, a splenectomy could be performed in conjunction with the fasciotomy. As the number of people infected with HIV increases, upper extremity surgeons may see more of this currently rare entity.
References Hey Groves EW. A clinical lecture upon the surgical aspects of hemophilia, with especial reference to two cases of Volkmann’s contracture resulting from this disease. Br Med J 1907;1:611-4. Lancourt JE, Gilbert M, Posner MA. Management of bleeding and associated complications of hemophilia in the hand forearm. J Bone Joint Surg 1977;59A: 4.51-60. Madigan RR, Hanna WT, Wallace SL. Acute compartment syndrome in hemophilia: a case report. J Bone Joint Surg 1981;63A:1327-9. Nixon RG, Brindley GW. Hemophilia presenting as
867
compartment syndrome in the arm following venipuncture: a case report and review of the literature. Clin Orthop 1989;244: 176-81. 5. Railton GT, Aronstam A. Early bleeding into upper limb muscles in severe hemophilia: clinical features and treatment. J Bone Joint Surg 1987;69B: 100-2. 6. Trumble T. Forearm compartment syndrome secondary to leukemic infiltrates. J Hand Surg 1987:12A: 563-5. pur7. Karpatkin S. Immunologic thrombocytopenic pura in HIV-seropositive homosexuals, narcotic addicts, and hemophiliacs. Semin Hematol 1988;25: 219-29. 8. Daugharty H, Chorba TL, Personette DW et al. Immunoglobulin bound to platelets as immune complexes or specific antibody in specimens from acquired immunodeficiency syndrome and immune thrombocytopenia purpura. Diagn Immunol 1985;3: 205- 14. 9. Glassman AB. Thrombocytopenia: proposed mechanisms and treatment in human immunodeficiency virus infection. Ann Clin Sci 1989;19:319-22. 10. Stroiker RB, Abrams DI, Corash LI, Shuman MA. Target platelet antigen in homosexual men with immune thrombocytopenia. N Engl J Med 1985:313: 1375-80. 11. Walsh CM, Nardi MA, Karpatkin S. On the mechanism of thrombocytopenic purpura in sexually active homosexual men. N Engl J Med 1984;311:635-9. of idiopathic 12. Lacey JV, Penner JA. Management thrombocytopenic purpura in the adult. Semin Thrombosis Hemostasis 1977;3: 160. 13. Hoffman DM, Caruso RF, Mirando T. Human immunodeficiency virus-associated thrombocytopenia. DICP 1989;23: 157-60. 14. Roy AJ, Jaffe N, Djerassi I. Prophylactic platelet transfusions in children with acute leukemia: a dose response study. Transfusion 1973:13:283-90. 1.5. Mubarak SJ. Compartment syndromes. In: Chapman MW, ed. Operative orthopaedics. New York: JB Lippincott. 1988: 179-95.
Acute compartment syndrome is most commonly associated with an upper extremity fracture; however, nontraumatic cases of acute forearm compartment syndrome associated with hemophilia’-’ and leukemic infiltrate@ have been documented. We describe a case of acute compartment syndrome following relatively minor trauma in a patient with human immunodeficiency virus (HIV)-associated thrombocytopenia.
Case Report A 25year-old Hispanic man, inmate at the county house of correction, bumped his left elbow and forearm lightly against a door 3 hours prior to presenting to the emergency department. He was complaining of moderate pain and swelling in the left forearm and elbow. He had no paresthesias at this time. He initially denied the use of any prescription or overthe-counter medications. Upon further questioning he admitted to prior intravenous drug use; an assay for antibody to the HIV had apparently been found to be positive 6 months prior to admission. He denied a history of joint pain, joint swelling, fever, cough, dysuria, rash, or unusual bleeding. On physical examination, he had significant swelling of the From the Department of Orthopaedics and Physical Rehabilitation, University of Massachusetts Medical Center and Department of Hematology, Medical Center of Central Massachusetts/ Memorial Hospital, Worcester, MA. Received for publication July 2, 1992; accepted in revised form March 15, 1993. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Sanjay S. Desai, MD, Department of Orthopaedics and Physical Rehabilitation, University of Massachusetts Medical Center, 55 Lake Avenue North, Worcester, MA 01655.
elbow and proximal forearm, but these were not tense. He had no pain on passive stretch of the fingers and had normal sensation to light touch in the median, ulnar, and radial nerve distribution in the left hand. There were no motor deficits in the left hand or forearm. He was afebrile. X-ray films revealed no evidence of fracture or dislocation in the elbow or forearm. Initial laboratory data were hematocrit, 38%; white blood cell count, 4900; and platelet count, 18,000. The prothrombin time was 12.1 seconds and the activated partial thromboplastin time was 41.6. The patient was admitted to the orthopaedic surgery service for splinting, frequent neurovascular checks, moderate elevation of the limb, and a hematology consultation. Approximately 18 hours after admission the patient began complaining of increased pain in the forearm and decreased sensation in the fingers of the left hand. On physical examination the forearm was noted to be more tense and the patient had increased pain on passive extension of the fingers. The anterior forearm compartment pressure, measured with a slit catheter, was in the 32-34 mmHg range. The patient was admitted to the surgical intensive care unit for closer monitoring and the slit catheter was left in place. Serial measurements over the next few hours showed anterior compartment pressures in the 28-30 mmHg range. The patient experienced some decrease in pain. Antinuclear antibody was found to be negative. A screen for disseminated intravascular coagulation was also negative, with a normal fibrinogen level and no elevation of fibrin split products. A screen for factor XIII level (clot solubility in 5 M urea) was also found to be normal. Because the patient was found to have a minimal prolongation of the partial thromboplastin time, factors VIII, IX, and XII were The Journal of Hand
Surgery
865
866
Desai et al.
i Acute Compartment
Syndrome
and HIV
measured and found to be normal. The factor XI level was found to be minimally decreased (54%). An equal mixture of patient and normal plasma demonstrated full correction of the activated partial thromboplastin time, arguing against the presence of a circulating inhibitor. The patient’s activated partial thromboplastin time returned to normal over the course of his stay in the hospital and remained normal thereafter. A presumptive diagnosis of HIV-related immune thrombocytopenia was made, and the patient was started on 60 mg of prednisone per day. Over the next 24 hours pain and paresthesias were markedly improved, and the last slit catheter pressure measured prior to discontinuing the catheter was 21 mmHg. The patient continued to improve over the next 3 days. At the time of discharge the patient’s platelet count was up to 142,000. He was discharged on 60 mg of prednisone per day. The patient had intermittent bouts of forearm swelling over the next several weeks, which correlated with waxing and waning of the platelet count. He required readmission for limb swelling but had no paresthesias or significant pain. In spite of continued steroid and Danazol therapy, the patient’s platelet count continued to be low. At short-term follow-up 6 weeks after his admission, he had some elbow stiffness but no evidence of ischemic contracture. Three months after his initial presentation he underwent splenectomy for refractory HIV-associated thrombocytopenia. He was lost to follow-up after this procedure.
Discussion Immune thrombocytopenia is a relatively common complication of intravenous drug use and can occur both in HIV-positive and HIV-negative subpopulations.’ The mechanism responsible for thrombocytopenia in this setting is unclear, although evidence has been found to implicate immune complexes’ and 7s IgG in the pathogenesis of narcoticassociated immune thrombocytopenic purpura.‘-” Compartment syndrome is a well-documented complication of hemophilia.‘-5 It is, however, an uncommon complication of qualitative or quantitative platelet disorders. For this reason, an extensive search for a second hemostatic defect was undertaken. The laboratory studies showed no evidence to support the presence of disseminated intravascular coagulation, coagulation factor deficiency, or a circulating anticoagulant. Definitive diagnosis in this case was hampered because the patient denied a history of intravenous drug abuse. However, consultation with a hematolo-
gist and further questioning of the patient yielded the correct diagnosis. The diagnosis of HIV-induced thrombocytopenia should be considered in young men with a low platelet count, whether or not the patient admits to any known risk factors. Platelet transfusion is generally not effective in raising the platelet count in immune-mediated thrombocytopenia. ‘* The main treatment modalities available include corticosteroids, intravenous immunoglobulin, Danazol, and splenectomy. The mechanism of action of these treatments is poorly understood. Currently accepted theories suggest that corticosteroids exert their effect in thrombocytopenia by reducing macrophage uptake of antibody-coated platelets by inhibiting F, receptor-mediated reticuloendothelial system function. Danazol is an androgenic steroid that is thought to decrease the number of F, receptors on monocytes and macrophages, thereby decreasing their ability to phagocytose altered platelets. Splenectomy eliminates a common site of platelet destruction.13 Most of these mechanisms are hypothetical. In this patient corticosteroids were useful as an initial measure to increase platelet count. It is possible that the patient’s symptoms improved spontaneously rather than due to the prednisone. However, throughout his subsequent clinical course there was a close correlation between his platelet count, forearm symptoms, and prednisone dose. That is, whenever his prednisone dose was decreased, his symptoms became worse and his platelet count decreased. In the long term, however, corticosteroids and Danazol were not effective in maintaining an adequate platelet count and the patient underwent splenectomy . We do not have unequivocal evidence that bleeding in the forearm was the cause of the patient’s symptoms. Short of surgery, magnetic resonance imaging may have been helpful in defining the pathology of increased compartment pressure in this situation. While there was minor trauma reported by the patient, his platelet count was below the accepted threshold (20,000) for spontaneous bleeding to occur. i4 While 30 mmHg is generally considered the threshold for fasciotomy in an acute compartment syndrome, it is a relative indication that must be tempered with the trend of symptoms and signs, as well as the trends of compartment pressures.i5 With steroids, rest, splinting, and mild elevation, the patient’s symptoms improved and compartment pressures showed a downward trend. This was fortunate as surgery on a thrombocytopenic patient, who may be actively bleeding, is a high-risk procedure. To our knowledge, this is the first reported case of
The Journal of Hand Surgery I Vol. 18A No. 5 September 1993
compartment syndrome related to HIV-associated thrombocytopenia. We recommend a high index of suspicion for HIV-induced thrombocytopenia in the patient with a history of minor trauma, compartment syndrome, and a low platelet count. We suggest prompt hematologic consultation and institution of medical therapy, along with rest, splinting, and monitoring of compartment pressures. We do not have experience in the surgical management of this entity, however, the compartment pressures and patient’s symptoms and signs could warrant surgical intervention. We recommend performing the fasciotomy in a standard fashion with a pneumatic tourniquet, meticulous hemostasis, and a bulky postoperative dressing with a splint. We also recommend close communication between the hand surgeon, hematologist, and general surgeon. When feasible, maximal pharmacologic therapy as outlined should be undertaken to increase the platelet count before fasciotomy. If necessary, a splenectomy could be performed in conjunction with the fasciotomy. As the number of people infected with HIV increases, upper extremity surgeons may see more of this currently rare entity.
References Hey Groves EW. A clinical lecture upon the surgical aspects of hemophilia, with especial reference to two cases of Volkmann’s contracture resulting from this disease. Br Med J 1907;1:611-4. Lancourt JE, Gilbert M, Posner MA. Management of bleeding and associated complications of hemophilia in the hand forearm. J Bone Joint Surg 1977;59A: 4.51-60. Madigan RR, Hanna WT, Wallace SL. Acute compartment syndrome in hemophilia: a case report. J Bone Joint Surg 1981;63A:1327-9. Nixon RG, Brindley GW. Hemophilia presenting as
867
compartment syndrome in the arm following venipuncture: a case report and review of the literature. Clin Orthop 1989;244: 176-81. 5. Railton GT, Aronstam A. Early bleeding into upper limb muscles in severe hemophilia: clinical features and treatment. J Bone Joint Surg 1987;69B: 100-2. 6. Trumble T. Forearm compartment syndrome secondary to leukemic infiltrates. J Hand Surg 1987:12A: 563-5. pur7. Karpatkin S. Immunologic thrombocytopenic pura in HIV-seropositive homosexuals, narcotic addicts, and hemophiliacs. Semin Hematol 1988;25: 219-29. 8. Daugharty H, Chorba TL, Personette DW et al. Immunoglobulin bound to platelets as immune complexes or specific antibody in specimens from acquired immunodeficiency syndrome and immune thrombocytopenia purpura. Diagn Immunol 1985;3: 205- 14. 9. Glassman AB. Thrombocytopenia: proposed mechanisms and treatment in human immunodeficiency virus infection. Ann Clin Sci 1989;19:319-22. 10. Stroiker RB, Abrams DI, Corash LI, Shuman MA. Target platelet antigen in homosexual men with immune thrombocytopenia. N Engl J Med 1985:313: 1375-80. 11. Walsh CM, Nardi MA, Karpatkin S. On the mechanism of thrombocytopenic purpura in sexually active homosexual men. N Engl J Med 1984;311:635-9. of idiopathic 12. Lacey JV, Penner JA. Management thrombocytopenic purpura in the adult. Semin Thrombosis Hemostasis 1977;3: 160. 13. Hoffman DM, Caruso RF, Mirando T. Human immunodeficiency virus-associated thrombocytopenia. DICP 1989;23: 157-60. 14. Roy AJ, Jaffe N, Djerassi I. Prophylactic platelet transfusions in children with acute leukemia: a dose response study. Transfusion 1973:13:283-90. 1.5. Mubarak SJ. Compartment syndromes. In: Chapman MW, ed. Operative orthopaedics. New York: JB Lippincott. 1988: 179-95.